Management of unclassifiable Interstitial Lung Diseases; A systematic review

Abstract

Unclassifiable interstitial lung diseases represent about 15% of all patients with interstitial lung disease diagnoses. Hence, it represents a significant proportion of patients, which requires a specialized team to treat. Unlike other types of interstitial lung diseases, it is difficult to confirm the diagnosis of unclassified interstitial lung diseases except with the help of a multidisciplinary team due to the patients' heterogeneity. This study aims at investigating the medical literature to assess the treatment strategies for unclassified interstitial lung diseases. The literature was examined through Medline, PubMed, Embase, and Ovid database in the duration from 2010 to 2020. Searching terms included were a combination of ‘’Management’’ OR‘’ Treatment’’ AND ‘unclassified interstitial lung diseases or ‘’UILD’’ AND ‘’outcome’’. Following this, results were refined to include only original research studies investigating the treatment strategies for unclassified interstitial lung diseases during the past decade. Selected trials mentioned the type of medication used as well as its outcome on treatment. A total of 589 studies were recovered. After the exclusion of articles on animals and including only trials on humans, 27 articles appeared. A total of seven articles were decided as eligible, covering a total of 1053 patients with unclassified interstitial lung diseases. Four studies were double-blind, randomized controlled trials, with three of them multi-center. One study was retrospective, and two studies were case reports. The review concluded that pirfenidone (monotherapy or in combination) and nintedanib are the two most promising medications for unclassified interstitial lung diseases. More studies with a robust design are required to examine potential intravenous cyclophosphamide and immune therapy treatment. Key-words: Unclassified Interstitial Lung Diseases; Management; Outcomes; Review